Sturge-Weber syndrome in port-wine stain patients: a retrospective study on the clinical features and screening strategy

Abstract

Objective: To investigate the clinical characteristics of Sturge-Weber syndrome (SWS) in the patients with Port-wine stain (PWS). Methods: A total of 279 PWS patients, 164 males, 115 females with a median age of first visit 17.00 (4.75, 56.00) months. Most of the PWS patients were referred to the Ophthalmology Department for screening eye problems when the cutaneous angiomas involved the ophthalmic division of the trigeminal nerve distribution. The intraocular pressure (IOP), cup to disk ratio (C/D), corneal condition and other essential measurements were examined to screen glaucoma or choroidal hemangioma. The differences of age, gender and vascular ectasia in the ipsilateral eyes were compared among PWS and SWS patients with chi-square test. The differences about the first visit time, IOP, C/D and corneal diameters were evaluated with independent-sample T test or nonparametric test followed by Mann-Whitney U test. Results: A total number of 66 out of 279 PWS patients (23.7%) were confirmed as SWS with glaucoma. The IOP of the ipsilateral eye with vascular ectasia in PWS and SWS was 13.00 mmHg (1 mmHg=0.133 kPa) (IQR: 9.75, 17.00) and 23.00 mmHg (20.00, 32.00), respectively (Z=-8.212, P<0.001); the IOP differences between the ipsilateral and contralateral eye in PWS and SWS was 1mmHg (0, 2) and 7 mmHg (3, 11) respectively; the C/D in the ipsilateral eye and the contralateral eye was 0.30 (0.30, 0.35) and 0.7 (0.6, 0.8) respectively in SWS cases with secondary glaucoma. Conclusions: There is a high proportion of SWS with glaucoma in ophthalmic division affected PWS patients. Fundus examinations were necessary for this type of patients. (Chin J Ophthalmol, 2017, 53:753-757).